
==== Front
Eur Heart J
Eur Heart J
eurheartj
European Heart Journal
0195-668X
1522-9645
Oxford University Press UK

39101600
10.1093/eurheartj/ehae485
ehae485
Cardiovascular Flashlights
AcademicSubjects/MED00200
Eurheartj/27
Eurheartj/29
Eurheartj/28
Pulsus alternans under left ventricular assist device in a patient with dilated cardiomyopathy and LMNA mutation
Sakai Ryohei Department of Cardiovascular Medicine, Niigata University Medical and Dental Hospital, 1-754 Asahimachidori, Chuoku, Niigata 951-8520, Japan

https://orcid.org/0000-0002-9267-1017
Kashimura Takeshi Department of Cardiovascular Medicine, Niigata University Medical and Dental Hospital, 1-754 Asahimachidori, Chuoku, Niigata 951-8520, Japan
Department of Advanced Cardiopulmonary Vascular Therapeutics, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachidori, Chuo-ku, Niigata 951-8510, Japan

Inomata Takayuki Department of Cardiovascular Medicine, Niigata University Medical and Dental Hospital, 1-754 Asahimachidori, Chuoku, Niigata 951-8520, Japan

Corresponding author. Tel: +81-25-227-2185, Fax: +81-25-227-0774, Email: kashi@med.niigata-u.ac.jp
14 9 2024
05 8 2024
05 8 2024
45 35 Focus Issue on Dyslipidaemias, Heart Failure and Cardiomyoptahies 32963296
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
2024
https://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
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pmcA 50-year-old man with dilated cardiomyopathy who had been treated with optimal medical therapy for 5 years and cardiac resynchronizing therapy with a defibrillator for 1 year for bradycardic atrial fibrillation presented with progressive heart failure with a left ventricular ejection fraction of 22% and skeletal muscle weakness. Left ventricular endomyocardial biopsy revealed distorted myocardial nuclei (Panel A) and interstitial fibrosis (Panel B), and blood sample analysis revealed a truncation mutation of LMNA (Panel C).

He underwent implantation of a left ventricular assist device (LVAD), Jarvik 2000, with an 8 s intermittent low-speed (ILS) mode in each 64 s period for facilitating aortic valve opening. The aortic valve opened with each heartbeat only during the ILS mode (Panels D and E).

However, 2 years later, echocardiography during the ILS mode showed alternating aortic valve opening or pulsus alternans (Panel F; Supplementary data online, Video S1). Two months later, the patient presented with dyspnoea and oedema; chest radiography revealed pleural effusion (Panel G). Right-sided heart failure progressed continuously, and the patient died 1 year later.

The mutation of LMNA, which encodes lamins A and C that localize to the inner nuclear membrane, may cause myocardial nuclear deformity and dysfunction.1 Pulsus alternans is presumably attributed to myocardial calcium–handling failure, which is one of the proposed mechanisms of myocardial dysfunction with LMNA mutation.2

Recently, pulsus alternans was reported in right-sided heart failure under peripheral LVAD in a patient with acute myocarditis.3 However, whether pulsus alternans under LVAD is a sign of right-sided heart failure or typical in LMNA mutation should be elucidated in future studies.

Supplementary data are available at European Heart Journal online.

All authors declare no disclosure of interest for this contribution.

No data were generated or analysed for or in support of this paper.

Supplementary Material

ehae485_Supplementary_Data
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References

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2 Morales Rodriguez  B, Domínguez-Rodríguez  A, Benitah  JP, Lefebvre  F, Marais  T, Mougenot  N, et al  Activation of sarcolipin expression and altered calcium cycling in LMNA cardiomyopathy. Biochem Biophys Rep  2020;22 :100767. 10.1016/j.bbrep.2020.100767 32490213
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